Ullrich-Turner syndrome associated with cystic medial necrosis of the aorta and great vessels

Cystic medial necrosis of the aorta with associated cardiovascular defects and complications is described in a patient with the Ullrich-Turner syndrome and a presumed XO constitution. The case and the literature are discussed with respect to the various manifestations of the congenital connective tissue defect which may occur in this syndrome.